Хроническая интестинальная псевдообструкция

Парфенов А.И., Крумс Л.М., Быкова С.В., Ахмадуллина О.В. Хроническая интестинальная псевдообструкция. Терапевтический архив. 2020; 92 (12): 4–9. DOI: 10.26442/00403660.2020.12.200426

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Parfenov A.I., Krums L.M., Bykova S.V., Ahmadullina O.V. Chronic intestinal pseudo-obstruction. Therapeutic Archive. 2020; 92 (12): 4–9. DOI:10.26442/00403660.2020.12.200426

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Аннотация

Хроническая интестинальная псевдообструкция (ХИПО) – редкое нарушение моторики желудочно-кишечного тракта, сходное с механической непроходимостью, при отсутствии анатомической обструкции. Развитие ХИПО обусловлено нарушением моторики гладких мышц и нервной системы желудочно-кишечного тракта. Общие симптомы включают запоры, боли в животе, тошноту, рвоту, вздутие живота. Нарушение перистальтики приводит к застою пищи в петлях тонкой кишки, их дилатации, развитию синдрома бактериального обсеменения. Нарушение питания и синдром бактериального обсеменения приводят к нарушению всасывания и кахексии. Лечение направлено на обеспечение адекватного питания, осуществление кишечной декомпрессии, применение препаратов, активизирующих моторику, подавление роста микробов в тонкой кишке. Хирургическое лечение: резекция пораженного сегмента кишки. При рефрактерном течении ХИПО – кишечная трансплантация.

Ключевые слова: хроническая интестинальная псевдообструкция, нейромиопатия, мальабсорбция, синдром бактериального обсеменения, парентеральное питание, прокинетики, кишечная декомпрессия, кишечная трансплантация.

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Chronic intestinal pseudo-obstruction a rare violation of the motor skills of the gastrointestinal complex, similar to mechanical obstruction, but without a mechanical obstacle. The development of chronic intestinal pseudo-obstruction is caused by a disturbance on the part of the smooth muscles and the nervous system of the gastrointestinal system. Common symptoms include constipation, abdominal pain, nausea, vomiting, bloating. Violation of peristalsis leads to food stagnation in the hinges of the small intestine, their dilation, the development of bacterial insemination syndrome. Eating disorders, bacterial contamination syndrome (CDDs) lead to impaired suction syndrome, cahexia. Treatment is aimed at providing adequate nutrition, the use of drugs that activate motor skills, suppress the growth of microbes in the small intestine, the implementation of intestinal decompression. Surgical treatment: resection of the affected segment of the gut. In the refractory course of the disease – intestinal transplantation.

Keywords: chronic intestinal pseudo-obstruction, neuromyopathy, malabsorption, bacterial insemination syndrome, parenteral nutrition, prokinetics, intestinal decompression, intestinal transplaniation.

Список литературы

1. Dudley HA, Sinctair IS, McLaren JE, et al. Intestinal psevdo –obstruction. J Roual Coll Surg Edinb.1958;3:206-17. doi: 10.1007/s11938-000-0047-2
2. Di Nardo G, Lorenzo CD, Lauro A, Stanghellini V, et al. Chronic intestinal pseudo- obstruction in children and adult: diagnosis and therapeutic options. Neurogastroenterik Motil. 2016;1:1-13. doi: 10.1111/nmo12945
3. Auricchio A, Brancolini V, Casari G, et al. The locus for novel sindromic form of neural intestinal pseudoobstruction maps to Xq28. Am J Hum Genet. 1996;58(4):747-8. PMID: 8644737.
4. FitzPatrick DR, Strain L, Thomas AE, et al. Neurogenic chronic idiopathic intestinal pseudo-obstruction, patent ductus arteriosus, and thrombocytopenia segregating as an X linked recessive disorder.
J Med Genet. 1997 Aug;34(8):666-9. doi: 10.1136/jmg.34.8.666
5. Guzé CD, Hyman PE, Payne VJ. Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome. Am J Med Genet. 1999 Jan 15;82(2):114-22. doi: 10.1002/(sici)1096-8628(19990115)82:2<114::aid-ajmg3>3.0.co;2-h
6. Miazga A, Osiriski M, Cichy W, et al. Current views on the etiopathogene, clinical manifestation, diagnostics, treatment and correlation with other nosological entities of SIBO. Adv Med Sci. 2015;60:118-24. doi: 10.1016/j.advms.2014.09.001. Epub 2014 Oct 6.
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11. Farrungia G. Intestinal cells in health and disease. Neurogastroenterol Motil. 2008;20 (Suppl. 1):54-63. doi: 10.1111/j.1365-2982.2008.01109.x
12. Wu JJ, Rothman TR, Gershon MD. Development of the interstitial cell of Cajal: Origin, Kit dependence and neuronal and nonneural sources of Kit Ligand. J Neurosci Res. 2000;59(3):384-401. doi: 10.1002/(SICI)1097-4547(20000201)59:3<384::AID-JNR13>3.0.CO;2-
13. De Giorgio R, Cogliandro R, Barbara G, et al. Chronic intestinal pseudo-obsrection: clinical features, diagnosis, and therapy. Gastroenterol Clin North Am. 2011;40:787-807. doi: 10.1016/j.gtc.2011.09.005
14. Di Baise J. Nutritional consequences of small intestinal bacterial overgrowth. Pract Gastroenterol. 2008;12:15-28. doi: 10.3748/wjg.v16.i24.2978
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16. Lapointe SP, Rivet C, Goulet O, et al. Urological manifestation, associated with chronic intestinal pseudo-obstruction in children. J Urol. 2002;158:1768-70. doi: 10.1097/01.ju.0000028495.91112.98
17. Pini Prato A, Rossi V, Fiore M, et al. Megacistic, megacolon, and malrotacion: a new syndromic association? Am J Med Genet. 2011;155A:1798-802. doi: 10.1002/ajmg.a.34119
18. Giordano C, Sebastiani M, De Giorgio R, et al. Gastrointestinal dysmotility in mitochondrial neurogastrointestinal encephalomyopathy is caused by mitochondrial DNA depletion. Am J Pathol. 2008;173:1120-8. doi: 10.2353/ajpath.2008.080252
19. Mann SD, Debinski HS, Kamm MA. Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults. Gut. 1997 Nov;41(5):675-81.
20. Amiot A, Tchikviladze V, Joly F, et al. Frequency of mitochondrial defects in patients with chronic intestinal pseudo-obstruction. Gastroenterology. 2009;137:101-9. doi: 10.1053/j.gastro.2009.03.054
21. Menys A, Butt S, Emmanuel A, et al. Comparative quantitative assessment of global small bowel motility using magnetic resonance imaging in chronic intestinal pseudo-obstruction and healthy controls. Neurogastroenterol Motil. 2016. Mar;28(3):376-83. doi: 10.1111/nmo.12735
22. Fell JME, Smith VV, Milla PJ. Infantile chronic intestinal pseudo-obstruction: The role of small intestinal manometry as diagnostic tool and prognostic indicator. Gut. 1996;39:306-11. doi: 10.1136/gut.39.2.306
23. Muñoz-Yagüe MT, Solís-Muñoz P, Salces I, et al. Chronic intestinal pseudo-obstruction: a diagnosis to be considered. Rev Esp Enferm Dig. 2009 May;101(5):336-42. doi: 10.4321/s1130-01082009000500005
24. Lindberg G, Tornblom H, Ivarson M, et al. Full-thickness biopsy finding in chronic intestinal pseudo-obstruction and dysmotility. Gut. 2009:58:1084-90. doi: 10.1136/gut.2008.148296
25. Muñoz-Yagüe MT, Martin JC, Colina F, Ibarrola C. Chronic primary intestinal pseudo-obstruction from visceral miopathy. Rev Esp Enferm Dig. 2006;98:292-302. doi: 10.4321/s1130-01082006000400007
26. Lebouvier T, Coron E, Chaumette T, et al. Routine colonic biopsies as a new tool to study the enteric nervous system in living patients. Neurogastroenterol Motil. 2010;22:11-4. doi: 10.1111/j.1365-2982.2009.01368.x
27. Lacy BE, Lowe BJ, Diagnosis, treatment and nutritional management of chronic intestinal pseudo-obstruction. Practical gastroenterology. 2009;9:9-24. doi: 10.1097/MPG.0000000000001608
28. Di Nardo G, Tarunarathe TV, Frediani S, De Giorgio R. Chronic intestinal pseudo-obstruction: Progress in management? Neurogastroenterol Motil. 2017;29:e13231. doi: 10.1111\nmo13231
29. Amiot A, Jolli F, Alves A, et al. Long- term outcome of chronic intestinal pseudo-obstruction adult patients requiring home parenteral nutrition. Am J Gastroenterol. 2009;104:1262-70. doi: 10.1038/ajg.2009.58
30. Emmanuel AV, Shand AG, Kamm MA. Eritromicin for treatment of chronic intestinal pseudo-obstruction.: description of six cases with positive response. Alim Pharmacol Ther. 2004;19(6):687-94. doi: 10.1007/s11938-005-0046-4
31. Rao AS, Camilleri M. Metoclopramid and tardive dyskinesia. Aliment Pharmacol Ther. 2010;31:11-9. doi: 10.1111/j.1365-2036.2009.04189.x
32. Soudah HC, Hasler WL, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma. N Engl J Med. 1991 Nov21;325(21):1461-7. doi: 10.1056/NEJM199111213252102
33. Emmanuel AV, Kamm MA, Roy AJ, et al. Randomised clinical trial: the efficacy of prucalopride in patients with chronic intestinal pseudo-obstruction – a double-blind, placebo-controlled, cross-over, multiple n = 1 study. Aliment Pharmacol Ther. 2012;35:48-55. doi: 10.1111/j.1365-2036.2011.04907.x
34. Gomez R, Fernandez S, Aspirot A, et al. Effect of amoxicillin/clavulanate on gastrointestinal motility in children. J Pediatr Gastroenterol Nutr. 2012 Jun;54(6):780-4. doi: 10.1097/MPG.0b013e31824204e4
35. Attar A, Kuoch V, Ducreux M, et al. Simultaneous decompression colonoscopy and radiologic G-tube insertion in a patient with megacolon because of chronic colonic pseudo-obstruction. Gastrointest Endosc. 2005 Dec;62(6):975-6; discussion 976. doi: 10.1016/j.gie.2005.06.058
36. Chun C, Aulakh S, Komlos E, Triadafilopoulos G. Tube to freedom: use of a venting jejunostomy in a patient with chronic intestinal pseudo-obstruction.. Dig Dis Sci. 2012;57:3076-9. doi: 10.1007/s10620-012-2111-x
37. Thompson AR, Pearson T, Ellul J, et al Percutaneous endoscopic colostomy in patients with chronic intestinal pseudo-obstruction. Gastrointest Endosc. 2004;59:113-5. doi: 10.1016/s0016-5107(03)02369-1
38. Sabbagh C, Amiot A, Maggiori L, et al. Nontransplantation surgical approach for chronic colonic pseudo-obstruction: analisis of 63 adult consecutive cases. Neurogastroenterol Motil. 2013;25:680-6. doi: 10.1111/nmo.12191
39. Lauro A, Zanfi C, Pellegrini S, et al. Isolated intestinal transplant for chronic intestinal pseudo-obstruction in adults: long-term outcome. Transplant Proc. 2013 Nov;45(9):3351-5. doi: 10.1016/j.transproceed.2013.06.014
40. Lauro A, Zanfi C, Dazzi A, et al. Disease-relate transplant in adults: result a single center. Transplant Proc. 2014;46:245-8. doi: 10.1016/j.transproceed.2013.08.110

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1. Dudley HA, Sinctair IS, McLaren JE, et al. Intestinal psevdo –obstruction. J Roual Coll Surg Edinb.1958;3:206-17. doi: 10.1007/s11938-000-0047-2
2. Di Nardo G, Lorenzo CD, Lauro A, Stanghellini V, et al. Chronic intestinal pseudo- obstruction in children and adult: diagnosis and therapeutic options. Neurogastroenterik Motil. 2016;1:1-13. doi: 10.1111/nmo12945
3. Auricchio A, Brancolini V, Casari G, et al. The locus for novel sindromic form of neural intestinal pseudoobstruction maps to Xq28. Am J Hum Genet. 1996;58(4):747-8. PMID: 8644737.
4. FitzPatrick DR, Strain L, Thomas AE, et al. Neurogenic chronic idiopathic intestinal pseudo-obstruction, patent ductus arteriosus, and thrombocytopenia segregating as an X linked recessive disorder.
J Med Genet. 1997 Aug;34(8):666-9. doi: 10.1136/jmg.34.8.666
5. Guzé CD, Hyman PE, Payne VJ. Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome. Am J Med Genet. 1999 Jan 15;82(2):114-22. doi: 10.1002/(sici)1096-8628(19990115)82:2<114::aid-ajmg3>3.0.co;2-h
6. Miazga A, Osiriski M, Cichy W, et al. Current views on the etiopathogene, clinical manifestation, diagnostics, treatment and correlation with other nosological entities of SIBO. Adv Med Sci. 2015;60:118-24. doi: 10.1016/j.advms.2014.09.001. Epub 2014 Oct 6.
7.[Parfenov AI. Chronic intestinal pseudoobstruction. In: Enterology. Moscow: MIA, 2009; p. 431-6 (In Russ.)].
8. De Giorgio R, Sarnelli G, Corinaldesi R, Stanghellini V. Advances in our understanding of the pathology chronic intestinal pseudo-obsruction. Gut. 2004;53:1549-52. doi: 10.1136/gut.2004.043968
9. Corinaldesi RF, Antonucci A, de Giorgio R, et al. Patient-repoted outcomes and gut dysmotility in functional gastrointestinal disorders. Neurogastroenterol Motil. 2011;23:1084-91. doi: 10.1111/j.1365-2982.2011.01783.x
10. [Niyazeva NV, Shchegolev AI, Marey MV, Suhih GT. Interstitial pacemaker cells. Bulletin of the RAMS. 2014;7-8:17-24 (In Russ.)].
11. Farrungia G. Intestinal cells in health and disease. Neurogastroenterol Motil. 2008;20 (Suppl. 1):54-63. doi: 10.1111/j.1365-2982.2008.01109.x
12. Wu JJ, Rothman TR, Gershon MD. Development of the interstitial cell of Cajal: Origin, Kit dependence and neuronal and nonneural sources of Kit Ligand. J Neurosci Res. 2000;59(3):384-401. doi: 10.1002/(SICI)1097-4547(20000201)59:3<384::AID-JNR13>3.0.CO;2-
13. De Giorgio R, Cogliandro R, Barbara G, et al. Chronic intestinal pseudo-obsrection: clinical features, diagnosis, and therapy. Gastroenterol Clin North Am. 2011;40:787-807. doi: 10.1016/j.gtc.2011.09.005
14. Di Baise J. Nutritional consequences of small intestinal bacterial overgrowth. Pract Gastroenterol. 2008;12:15-28. doi: 10.3748/wjg.v16.i24.2978
15. Yeung Ak, Di Lorenzo C. Primery gastrointestinal motility disorders in childhood. Minerva Pediatr. 2012;64:567-84. PMID: 23108319.
16. Lapointe SP, Rivet C, Goulet O, et al. Urological manifestation, associated with chronic intestinal pseudo-obstruction in children. J Urol. 2002;158:1768-70. doi: 10.1097/01.ju.0000028495.91112.98
17. Pini Prato A, Rossi V, Fiore M, et al. Megacistic, megacolon, and malrotacion: a new syndromic association? Am J Med Genet. 2011;155A:1798-802. doi: 10.1002/ajmg.a.34119
18. Giordano C, Sebastiani M, De Giorgio R, et al. Gastrointestinal dysmotility in mitochondrial neurogastrointestinal encephalomyopathy is caused by mitochondrial DNA depletion. Am J Pathol. 2008;173:1120-8. doi: 10.2353/ajpath.2008.080252
19. Mann SD, Debinski HS, Kamm MA. Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults. Gut. 1997 Nov;41(5):675-81.
20. Amiot A, Tchikviladze V, Joly F, et al. Frequency of mitochondrial defects in patients with chronic intestinal pseudo-obstruction. Gastroenterology. 2009;137:101-9. doi: 10.1053/j.gastro.2009.03.054
21. Menys A, Butt S, Emmanuel A, et al. Comparative quantitative assessment of global small bowel motility using magnetic resonance imaging in chronic intestinal pseudo-obstruction and healthy controls. Neurogastroenterol Motil. 2016. Mar;28(3):376-83. doi: 10.1111/nmo.12735
22. Fell JME, Smith VV, Milla PJ. Infantile chronic intestinal pseudo-obstruction: The role of small intestinal manometry as diagnostic tool and prognostic indicator. Gut. 1996;39:306-11. doi: 10.1136/gut.39.2.306
23. Muñoz-Yagüe MT, Solís-Muñoz P, Salces I, et al. Chronic intestinal pseudo-obstruction: a diagnosis to be considered. Rev Esp Enferm Dig. 2009 May;101(5):336-42. doi: 10.4321/s1130-01082009000500005
24. Lindberg G, Tornblom H, Ivarson M, et al. Full-thickness biopsy finding in chronic intestinal pseudo-obstruction and dysmotility. Gut. 2009:58:1084-90. doi: 10.1136/gut.2008.148296
25. Muñoz-Yagüe MT, Martin JC, Colina F, Ibarrola C. Chronic primary intestinal pseudo-obstruction from visceral miopathy. Rev Esp Enferm Dig. 2006;98:292-302. doi: 10.4321/s1130-01082006000400007
26. Lebouvier T, Coron E, Chaumette T, et al. Routine colonic biopsies as a new tool to study the enteric nervous system in living patients. Neurogastroenterol Motil. 2010;22:11-4. doi: 10.1111/j.1365-2982.2009.01368.x
27. Lacy BE, Lowe BJ, Diagnosis, treatment and nutritional management of chronic intestinal pseudo-obstruction. Practical gastroenterology. 2009;9:9-24. doi: 10.1097/MPG.0000000000001608
28. Di Nardo G, Tarunarathe TV, Frediani S, De Giorgio R. Chronic intestinal pseudo-obstruction: Progress in management? Neurogastroenterol Motil. 2017;29:e13231. doi: 10.1111\nmo13231
29. Amiot A, Jolli F, Alves A, et al. Long- term outcome of chronic intestinal pseudo-obstruction adult patients requiring home parenteral nutrition. Am J Gastroenterol. 2009;104:1262-70. doi: 10.1038/ajg.2009.58
30. Emmanuel AV, Shand AG, Kamm MA. Eritromicin for treatment of chronic intestinal pseudo-obstruction.: description of six cases with positive response. Alim Pharmacol Ther. 2004;19(6):687-94. doi: 10.1007/s11938-005-0046-4
31. Rao AS, Camilleri M. Metoclopramid and tardive dyskinesia. Aliment Pharmacol Ther. 2010;31:11-9. doi: 10.1111/j.1365-2036.2009.04189.x
32. Soudah HC, Hasler WL, Owyang C. Effect of octreotide on intestinal motility and bacterial overgrowth in scleroderma. N Engl J Med. 1991 Nov21;325(21):1461-7. doi: 10.1056/NEJM199111213252102
33. Emmanuel AV, Kamm MA, Roy AJ, et al. Randomised clinical trial: the efficacy of prucalopride in patients with chronic intestinal pseudo-obstruction – a double-blind, placebo-controlled, cross-over, multiple n = 1 study. Aliment Pharmacol Ther. 2012;35:48-55. doi: 10.1111/j.1365-2036.2011.04907.x
34. Gomez R, Fernandez S, Aspirot A, et al. Effect of amoxicillin/clavulanate on gastrointestinal motility in children. J Pediatr Gastroenterol Nutr. 2012 Jun;54(6):780-4. doi: 10.1097/MPG.0b013e31824204e4
35. Attar A, Kuoch V, Ducreux M, et al. Simultaneous decompression colonoscopy and radiologic G-tube insertion in a patient with megacolon because of chronic colonic pseudo-obstruction. Gastrointest Endosc. 2005 Dec;62(6):975-6; discussion 976. doi: 10.1016/j.gie.2005.06.058
36. Chun C, Aulakh S, Komlos E, Triadafilopoulos G. Tube to freedom: use of a venting jejunostomy in a patient with chronic intestinal pseudo-obstruction.. Dig Dis Sci. 2012;57:3076-9. doi: 10.1007/s10620-012-2111-x
37. Thompson AR, Pearson T, Ellul J, et al Percutaneous endoscopic colostomy in patients with chronic intestinal pseudo-obstruction. Gastrointest Endosc. 2004;59:113-5. doi: 10.1016/s0016-5107(03)02369-1
38. Sabbagh C, Amiot A, Maggiori L, et al. Nontransplantation surgical approach for chronic colonic pseudo-obstruction: analisis of 63 adult consecutive cases. Neurogastroenterol Motil. 2013;25:680-6. doi: 10.1111/nmo.12191
39. Lauro A, Zanfi C, Pellegrini S, et al. Isolated intestinal transplant for chronic intestinal pseudo-obstruction in adults: long-term outcome. Transplant Proc. 2013 Nov;45(9):3351-5. doi: 10.1016/j.transproceed.2013.06.014
40. Lauro A, Zanfi C, Dazzi A, et al. Disease-relate transplant in adults: result a single center. Transplant Proc. 2014;46:245-8. doi: 10.1016/j.transproceed.2013.08.110

Авторы

А.И. Парфенов, Л.М. Крумс, С.В. Быкова, О.В. Ахмадуллина

ГБУЗ «Московский клинический научно-практический центр им. А.С. Логинова» Департамента здравоохранения г. Москвы,
Москва, Россия

________________________________________________

A.I. Parfenov, L.M. Krums, S.V. Bykova, O.V. Ahmadullina

Loginov Moscow Clinical Scientific Center, Moscow, Russia

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